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DOI: 10.4103/0976-237X.68588

Herpes zoster oticus: A rare clinical entity

SHAILESH GONDIVKAR, VIREN PARIKH1, RIMA PARIKH1

Abstract
Herpes zoster oticus also known as Ramsay Hunt syndrome is a rare complication of herpes zoster in which reactivation of
latent varicella zoster virus infection in the geniculate ganglion causes otalgia, auricular vesicles, and peripheral facial paralysis.
Ramsay Hunt syndrome is rare in children and affects both sexes equally. Incidence and clinical severity increases when host
immunity is compromised. Because these symptoms do not always present at the onset, this syndrome can be misdiagnosed.
Although secondary to Bells palsy in terms of the cause of acute atraumatic peripheral facial paralysis, Ramsay Hunt syndrome,
with incidence ranged from 0.3 to 18%, has a worse prognosis. Herpes zoster oticus accounts for about 12% cases of facial palsy,
which is usually unilateral and complete and full recovery occurs in only about 20% of untreated patients. The most advisable
method to treat Ramsay Hunt syndrome is the combination therapy with acyclovir and prednisone but still not promising, and
several prerequisites are required for better results. We present a case of 32-year-old man suffering from Ramsay Hunt syndrome
with grade V facial palsy treated effectively with rehabilitation program, after the termination of the combination therapy of acyclovir
and prednisone.

Keywords: Geniculate ganglion, facial palsy, otalgia, Ramsay Hunt syndrome, unilateral

Introduction in past 6 months before the onset of rash. On examination,

Ramsay Hunt syndrome (RHS), first described by James
Ramsay Hunt in 1907,[1] is caused by reactivation of VZV which
lies latent in sensory root ganglion for years in a patient who
had chickenpox earlier. Involvement of geniculate ganglion of
sensory branch of facial nerve leads to herpes zoster oticus
(HZO) also known as RHS. Involvement of facial nerve leads
to otalgia, lower motor neuron homolateral facial paralysis,
and vesicular eruptions in auricle. In severe cases of HZO,
involvement of vestibulocochlear nerve leads to sensorineural
hearing loss in 10% and vestibular symptoms in 40% patients.
Definitive treatment consists of antiviral therapy and steroids.
This article describes the case of RHS with grade V facial palsy
of House-Brackmann grading system treated effectively with
combination therapy of acyclovir and prednisone, supported
by rehabilitation program.
Case Report
A 32-year-old man with previous healthy condition presented
to us with pain in left ear for last 2 days. 24 to 36 h after the
onset of otalgia, patient developed facial weakness along
with vesicular eruptions on conchae and in external auditory
meatus of left side. There was history of stressful life events
Department of Oral Diagnosis, Medicine and Radiology, K. M.
Shah Dental College and Hospital, Piparia, Vadodara, Gujarat,
1
Parikh Hospital, Veraval, Gujarat, India
Correspondence: Dr. Shailesh Gondivkar,
# 21, Madhuban, Shakuntal colony, VMV Road,
Amravati - 444 604, Maharashtra, India
E-mail: shailesh_gondivkar@yahoo.com
there was lower motor neuron facial palsy on left side which
was complete. Bells phenomenon was present on left side
[Figure 1]. A neurologic examination revealed a weakness
in the marginal mandibular branch of the left facial nerve
[Figure 2]. Loss of definition in the ipsilateral nasolabial fold
[Figure 1] and weakness in the temporal branch of the facial
nerve was detected. There were painful adherent crusts and
scabs in left conchae and external auditory meatus [Figure 3],
associated with unclear hearing of left ear. The oral cavity and
oropharynx were normal. Ocular examination demonstrated a
spontaneous, lateral, left-beating gaze nystagmus but normal
conjunctiva and sclera.
The patient was seen by an ENT consultant and the diagnosis
of RHS with grade V facial palsy was confirmed. The ENT
consultant administered an initial dose of intravenous
acyclovir and steroids, and discharged the patient with a
2-week course of oral acyclovir and steroids. He was also
referred to an ophthalmology clinic for corneal assessment.
Meanwhile, because of the persistent grade V facial palsy,
rehabilitation therapy was requested.
The rehabilitation program that the patient attended included
transcutaneous electrical nerve stimulation and facial
neuromuscular exercises. The facial exercise program was
composed of (1) relaxation of hyperactive muscles, (2) facial
massage exercises, (3) biofeedback training using a mirror
to let patient know facial movement, and (4) specific facial
exercises like smiling, grimacing, and whistling. The patient
was instructed by the physiotherapist to follow this exercise
program for two times a week, at least 60 min per visit and
encouraged him to do methodically the facial exercise himself
in front of a mirror at home. It took additional 3 weeks of
outpatient rehabilitation program for total remission of his

127 Contemporary Clinical Dentistry | Apr-Jun 2010 | Vol 1| Issue 2

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Gondivkar, et al.: Herpes zoster oticus
Figure 1: Photograph showing the presence of Bells
phenomenon and obliteration of nasolabial fold on left side
Figure 2: Photograph while full smiling
Figure 3: Photograph showing crusted eruptions on left
conchae and external auditory meatus
facial palsy to occur, in addition to the 1-week inpatient
rehabilitation.
Contemporary Clinical Dentistry | Apr-Jun 2010 | Vol 1| Issue 2
Table 1: Clinical features of Ramsay Hunt syndrome
Key Acute peripheral facial paralysis
Clinical Vesicles occurring anywhere along the
features sensory distribution of the facial nerve,
including the anterior two-thirds of the tongue,
the pinna or the external auditory canal
Otalgia
Additional Tinnitus
clinical features Hearing loss
Vertigo
Nausea
Vomiting
Nystagmus
Change in taste perception
Discussion
Ramsay Hunt suggested that HZO was due to geniculate
ganglionitis. However, many contemporary authors agree
that this condition represents a polycranial neuronitis. Several
observations concluded that the primary etiologic agent of
RHS is VZV but Bells palsy, in contrast, has been attributed
to herpes simplex virus type-1.[2]
Herpes zoster is seen as a disease of older people (most
commonly over 60 years old), and incidence and severity
increases with age which may be due to a decline in cellular
rather than humoral immunity.[3] The VZV reactivation in the
geniculate ganglia and subsequent neural inflammation,
pressure, and possible destruction of the facial nerve in the
temporal bone are suspected to cause facial palsy,[1] while
VZV migrates from the geniculate ganglia into the skin
around the ear or into the oropharynx via the sensory fibers,
where it replicates and produces zoster in RHS.[1] Frequently,
there is involvement of VIII cranial nerve producing hearing
impairment and vertigo. Involvement of cranial nerves V, IX,
X, XI, and XII occurs less frequently.
Facial palsy and zoster do not always appear simultaneously,
and some patients with RHS exhibit facial palsy several days
before or after the onset of zoster. VZV also causes acute
peripheral facial palsy with the absence of skin lesions;
such cases are termed zoster sine herpete and are usually
diagnosed using serological assays[4] or polymerase chain
reaction (PCR).[5] The diagnosis of RHS is based on the history
and clinical findings mentioned in Table 1. Oral lesions are
also present in most cases. Laboratory confirmation of the
clinical diagnosis is based on increasing antibody titer in
repeated complement fixation tests. PCR can detect VZV in
saliva, tears, middle ear fluid, and blood mononuclear cells.[5]
The most recommended therapy for RHS is the combination
of acyclovir and prednisone.[6] Acyclovir is an effective
antimicrobial agent against actively replicating herpes
zoster viruses. Acyclovir itself is not active. It must first
be phosphorylated by viral thymidine kinase to form a
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Gondivkar, et al.: Herpes zoster oticus
triphosphate. Acyclovir triphosphate inhibits viral DNA
polymerase and, thus, DNA replication. [7] Importantly,
no statistically significant outcome differences were
noted between patients treated with intravenous or oral
acyclovir.[7] Because of increasing viral resistance to acyclovir,[7]
newer drugs, such as valacyclovir, famciclovir, penciclovir and
brivudine, are being more commonly used.
Adjunctive steroid therapy can be helpful in the management
of the facial paralysis of RHS.[8] A study on 80 RHS patients
with different levels of severity treated with acyclovir-
prednisone combination showed complete facial recovery,
i.e., House grade I, in 52% patients, no matter what their
pretreatment gradings were.[6]
For a significant improvement of facial recovery and hearing
loss, early combination therapy within 3 days of the onset of
facial palsy was critical; chances of grade I recovery was less
than 30% if therapy started later than 7 days of onset. Previous
studies reported better recovery of facial nerve function with
combination therapy of acyclovir and steroids than steroids
alone.[8] However, many authors caution against implementing
steroid therapy, especially with periocular lesions, as they
fear dissemination of the varicella zoster virus infection.[9]
The rehabilitation program for facial palsy includes electrical
stimulation, infrared radiation, and facial neuromuscular
exercises including automassage, relaxation exercises,
inhibition of synkinesis, co-ordination exercises, or emotional
expression exercises. The immunization of older persons with
a VZV vaccine would boost their cell-mediated immunity to
129
VZV and thereby provide protection against herpes zoster
and postherpetic neuralgia.[10]
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Source of Support: Nil, Conflict of Interest: None declared.
Contemporary Clinical Dentistry | Apr-Jun 2010 | Vol 1| Issue 2